Baby born with heart outside her body is making strides

Updated 9:05 am, Thursday, January 24, 2013

Photo: Texas Children's Hospital

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A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’ condition was discovered during an ultrasound 16 weeks into her mother's pregnancy. The mother, from Midland, Texas, was referred to Texas Children's, where a team of doctors worked together on her care after she was born.

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’ condition was discovered during an ultrasound 16 weeks into her mother's pregnancy. The mother, from Midland, Texas, was referred to Texas Children's, where a team of doctors worked together on her care after she was born.

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’ condition was discovered during an ultrasound 16 weeks into her mother's pregnancy. The mother, from Midland, Texas, was referred to Texas Children's, where a team of doctors worked together on her care after she was born.

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’

Ashley Cardenas, holds the finger of her daughter, Audrina Cardenas, five weeks old, who is recovering at Texas Children's Hospital after being was born with her heart outside her body Tuesday, Nov. 20, 2012, in Houston. Surgeons at Texas Children's Hospital preformed a life-saving surgery five weeks ago to repair Audrina's heart and she is now the longest surviving baby recorded in Texas to be born with this condition.

Ashley Cardenas, holds the finger of her daughter, Audrina Cardenas, five weeks old, who is recovering at Texas Children's Hospital after being was born with her heart outside her body Tuesday, Nov. 20, 2012,

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’ condition was discovered during an ultrasound 16 weeks into her mother's pregnancy. The mother, from Midland, Texas, was referred to Texas Children's, where a team of doctors worked together on her care after she was born.

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’

Ashley Cardenas, left, and her sister, Raquel Cardenas, look at Ashley's daughter, Audrina Cardenas, five weeks old, who is recovering at Texas Children's Hospital after being was born with her heart outside her body Tuesday, Nov. 20, 2012, in Houston. Surgeons at Texas Children's Hospital preformed a life-saving surgery five weeks ago to repair Audrina's heart and she is now the longest surviving baby recorded in Texas to be born with this condition.

Ashley Cardenas, left, and her sister, Raquel Cardenas, look at Ashley's daughter, Audrina Cardenas, five weeks old, who is recovering at Texas Children's Hospital after being was born with her heart outside

Audrina Cardenas, five weeks old, is recovering at Texas Children's Hospital after being was born with her heart outside her body Tuesday, Nov. 20, 2012, in Houston. Surgeons at Texas Children's Hospital preformed a life-saving surgery five weeks ago to repair Audrina's heart and she is now the longest surviving baby recorded in Texas to be born with this condition.

Audrina Cardenas, five weeks old, is recovering at Texas Children's Hospital after being was born with her heart outside her body Tuesday, Nov. 20, 2012, in Houston. Surgeons at Texas Children's Hospital

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’ condition was discovered during an ultrasound 16 weeks into her mother's pregnancy. The mother, from Midland, Texas, was referred to Texas Children's, where a team of doctors worked together on her care after she was born.

A baby girl who was born with her heart outside of her body is defying the odds and recovering after lifesaving surgery at Texas Children's Hospital, where she was born five weeks ago. Audrina Cardenas’

An infant girl born with all the right parts but a key one in the wrong place is recovering at Texas Children's Hospital, five weeks after she was delivered with one of the rarest of heart defects.

Texas Children's doctors reported Tuesday on the remarkable case of Audrina Cardenas, who was born with much of her heart outside her body, a highly unusual condition that's usually fatal within the first few days after birth. The doctors said her prognosis is favorable.

"I think she's going to make it," said Dr. Charles Fraser, the Texas Children's surgeon-in-chief who led the surgery. "Her heart's structurally in good shape, and she's survived the initial 48 to 72 hours after birth that are so tenuous for these patients."

Fraser and his team of cardiovascular, pediatric and plastic surgeons performed a six-hour open-heart surgery the day after Audrina's delivery Oct. 15, fashioning a cavity in the chest, putting the protruding heart in it and then stretching skin from elsewhere on her body over it. You can see video of the baby's heart beating here. (Warning: video is graphic)

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The heart, which was conspicuously visible outside the infant's abdomen at birth, now doesn't protrude, even under the skin, Fraser said.

The surgeon, also a Baylor College of Medicine professor, said the fragility of infants' chests makes the surgery particularly challenging. He added that a heart that's unprotected by the breastplate and surrounding tissue and exposed to the outside world is very vulnerable to infections, which is a common cause of death.

Doctors ultimately will need to fashion a breastplate around Audrina's heart, Fraser said.

Mortality 90 percent

Normally, the heart cavity and breastplate develop naturally.

Audrina's condition, known as ectopia cordia, occurs in six to eight of every 1 million babies. Doctors don't know the condition's cause, but about 90 percent of babies born with it are either stillborn or die within the first three days of life.

Excruciating choice

Audrina's condition was discovered 16 weeks into the pregnancy of Ashley Cardenas, 25, an Odessa title loans manager and mother of twins. She'd never heard of the diagnosis when doctors told her an ultrasound showed the developing malformation.

Cardenas was referred to Texas Children's but not before her doctors gave her three options: abort; bring the baby to term, but then provide only comfort care to ease any suffering before she died; or put her through the difficult, often unsuccessful surgery.

At the time, doctors didn't know Audrina's chances, whether she was a good candidate to survive or - more likely - to be stillborn or die soon after birth.

Acknowledging the difficulty of the decision, Cardenas said, "You'll never know what it feels like to make that decision until you're in that situation yourself."

She said prayer and the support of family and friends helped her decide.

Dr. Carolyn Altman, a pediatric cardiologist at Texas Children's and Baylor Med who was part of the team, said she saw signs of hope when she viewed ultrasound images of Audrina.

"When I first heard we had an ectopia cardis patient, I got a pit in my stomach because I know how difficult they are and because the outcome is usually so bad," Altman admitted. But after the ultrasound scan, she said, "I thought, 'This little one might actually have a chance.' Only about a third of the heart was outside the body and there was none of the bad congenital problems you usually see. I thought this was one we could actually help survive."

Altman said that life-threatening, complex heart disease occurs in about 60 percent of ectopia cordis cases.

'Better every day'

Audrina will need more surgery to correct relatively minor heart defects, but her doctors hope to wait at least a couple of years to give her time to heal and develop. The defects, involving two holes and an obstruction in chambers of the heart, are not uncommon in babies and are treatable.

Audrina remains in intensive care, still on a ventilator, but not needing any other support. She is on normal nutrition.

For now, Cardenas has had to be content with holding "her little hand, putting a head band on her, that sort of thing," because she hasn't yet been allowed to hold Audrina in the ICU.